Endoscopic endonasal approach for loco-regional recurrent clivus chordomas

Abstract

•EEA represents an ideal approach for loco-regional recurrent CCs.•EEA is well tolerated, with preservation of patients QoL.•EEA can be considered for patients with perspectives of adjuvant therapies.•Otherwise, EEA can be considered only in selected cases with a palliative aim.

Fig. 1

Intra-operative endoscopic image, 0° scope. Approach phase. A, B and C It is important to harvest the naso-septal flap as soon as possible, especially in patients already operated via EEA, to avoid any damage to potentially relevant homologous materials for the closure at the end of surgery. In this case, the mucoperiustium and its pedicle at the right side of the septum were kept intact from previous surgery and this flap still was available. D. After its preparation, the flap can be located in the rhinopharynx or in the maxillary sinus, and the previous surgical approach can be enlarged to expose the tumor as larger as possible.

Fig. 2

Intra-operative endoscopic image, 0° scope. Tumor removal. AA. The extracranial portion of the tumor can be resected with the debrider, which is particularly effective also in presence of scars from previous surgeries. B. In its intracranial portion, it is preferable to remove the tumor with curretes and suction, to reduce the risk of surgical complication. Ultrasonic aspirator can be also used, especially for firm CC. C and D. During tumor removal, it is necessary to check the location of the carotid arteries by neuronavigation (C) and intra-operative Doppler (D), reducing the risk of injuries to these vessels.

Fig. 3

Intra-operative endoscopic image, 0° and 30° scope. Tumor removal. A. After resection of the extradural portion of tumor, the CC can be followed in the intradural space. B. The dura is cut, and the tumor progressively mobilized. C and D. Inspection of the surgical field at the end of surgery both with 0° (C) and 30° (D) scope, to detect any possible tumor remnants.

Fig. 4

Intra-operative endoscopic image, 0° scope. Closure. If no CSF leak was observed, the surgical cavity is filled with fat and eventually covered with mucoperiosteum, especially if carotid arteries have been exposed to avoid delayed ruptures. A. In case of CSF leak, closure includes an intradural layer with a heterologous dural substitute. B. Afterwards, fat is placed to fill the cavity. C. The closure is covered with the mucoperiosteum flap, harvested at the beginning of surgery. Whether no pedicled flaps is available, it should be considered the use of alternative techniques, as galea or temporalis fascia flap, depending on tumor extension.

Fig. 5

MRI, an exemplificative case of loco-regional recurrent chordoma, underwent surgery with a resective aim is reported. The patient of 32 years old was already treated by an EEA, followed by heavy particles radiotherapy. After 42 months, he presented with loco-regional recurrence, located in the middle/inferior third of the clivus with extra- and intra-dural extension, causing diplopia for CN VI palsy. A and B. T1-w with gadolium, sagittal and coronal view, showing the recurrent tumor. An EEA was planned with the aim to remove the tumor, followed by a second radiation therapy. C and D. T1-w with gadolium, sagittal and coronal view, demonstrating the complete tumor removal. Diplopia regressed and the patient is still alive at follow-up of 24 months, without any further tumor progression.

Fig. 6

MRI, an exemplificative case of loco-regional recurrent chordoma, underwent surgery with a palliative aim is reported. The patient of 62 years old was already treated by one transcranial approach and one EEA, followed by one radiosurgery and heavy particles radiotherapy. After 24 months, he presented with a progression of the previous remnant, located in the middle/inferior third of the clivus with extra- and intra-dural extension, causing nasal obstruction, trigeminal pain resistant to medications and diplopia. A and B. T1-w with gadolium, sagittal and coronal view, showing the recurrent tumor. An EEA was planned with the aim to decompress the neural structures to alleviate the patients symptoms. C and D. T1-w with gadolium, sagittal and coronal view, demonstrating the partial tumor resection. At follow-up, the trigeminal nevralgia was controlled by medical therapy and diplopia and nasal obstruction regressed. The patient was addressed to the palliation care unit, and he died 18 months later.

Fig. 7

Kaplan-Meier analyses for OS. A. OS for primary and loco-regional recurrent cases. B. OS for primary and loco-regional recurrent cases, considering separately those cases underwent surgery with a resective and palliative aim. Time is expressed in days.

Fig. 8

Kaplan-Meier analyses for PFS. A. PFS for primary and loco-regional recurrent cases. B. PFS for primary and loco-regional recurrent cases, considering separately those cases underwent surgery with a resective and palliative aim. Time is expressed in days.

Fig. 9

Kaplan-Meier analyses to for the factors influencing the PFS in loco-regional recurrent chordomas. A. PFS for tumors already undergone to radiotherapy or naive for radiotherapy demonstrates a protective effect of radiation therapy. B. PFS for tumors with a size larger or inferior to 3 ​cm. This parameter resulted not associated to a difference in PFS at Kaplan-Meier analysis. Time is expressed in days.